製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号Q53GS7
    • 生物種Human
    • 配列RMKGTEGLRLWQEEQERKVQALSEMASEQLKRFDEWKELKQHKEFQDLRE VMEKSSREALGHQEKLKAEHRHRAKILNLKLREAEQQRVKQAEQERLRKE E
    • 分子量37 kDa including tags
    • 領域140 to 240

特性

Our Abpromise guarantee covers the use of ab114682 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • アプリケーション

    Western blot

    SDS-PAGE

    ELISA

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

関連情報

  • 別名
    • GLE 1
    • GLE1
    • GLE1 like protein
    • GLE1 like RNA export mediator
    • GLE1 RNA export mediator homolog
    • GLE1 RNA export mediator like (yeast)
    • GLE1-like protein
    • GLE1_HUMAN
    • GLE1L
    • hGLE1
    • LCCS
    • LCCS 1
    • LCCS1
    • Nucleoporin GLE1
    see all
  • 機能Required for the export of mRNAs containing poly(A) tails from the nucleus into the cytoplasm. May be involved in the terminal step of the mRNA transport through the nuclear pore complex (NPC).
  • 関連疾患Defects in GLE1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1) [MIM:253310]; also known as multiple contracture syndrome type Finnish. LCCS is an autosomal recessive disorder characterized by early fetal hydrops and akinesia, micrognatia, pulmonary hypoplasia, pterygia, multiple joint contractures, specific neuropathology with degeneration of anterior horn neurons and extreme skeletal muscle atrophy. LCCS1 leads to prenatal death.
    Defects in GLE1 are the cause of lethal arthrogryposis with anterior horn cell disease (LAAHD) [MIM:611890]. LAAHD is characterized by fetal akinesia, arthrogryposis and motor neuron loss. LAADH fetus often survive delivery, but die early as a result of respiratory failure. Neuropathological findings resemble those of LCCS1, but are less severe.
  • 配列類似性Belongs to the GLE1 family.
  • 細胞内局在Nucleus. Cytoplasm. Shuttles between the nucleus and the cytoplasm. Shuttling is essential for its mRNA export function and Cytoplasm. Nucleus > nuclear pore complex. Shuttles between the nucleus and the cytoplasm. In the nucleus, isoform 1 localizes to the nuclear pore complex and nuclear envelope. Shuttling is essential for its mRNA export function.
  • Information by UniProt

Recombinant Human GLE1 protein 画像

  • ab114682 analysed on a 12.5% SDS-PAGE gel stained with Coomassie Blue.

Recombinant Human GLE1 protein (ab114682) 使用論文

ab114682 has not yet been referenced specifically in any publications.

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